Hunter syndrome: ndi chiyani, matenda, zizindikiro ndi chithandizo

Zamkati

• Zizindikiro za Hunter Syndrome
• Momwe matendawa amapangidwira
• Chithandizo cha Hunter Syndrome

Hunter Syndrome, yemwenso amadziwika kuti Mucopolysaccharidosis mtundu wachiwiri kapena MPS II, ndi matenda osowa obadwa nawo omwe amapezeka kwambiri mwa amuna omwe amadziwika ndi kusowa kwa enzyme, Iduronate-2-Sulfatase, yomwe ndiyofunikira pakugwira bwino ntchito kwa thupi.

Chifukwa chakuchepa kwa ntchito ya enzyme iyi, pamakhala kuchuluka kwa zinthu mkati mwa maselo, zomwe zimabweretsa zizindikilo zowopsa komanso kusintha kosinthika, monga kuuma kwamalumikizidwe, mtima ndi kusintha kwa kupuma, mawonekedwe a zotupa pakhungu ndikusintha kwamitsempha, mwachitsanzo .

Zizindikiro za Hunter Syndrome

Zizindikiro za Hunter Syndrome, kufulumira kwa matenda komanso kuopsa kwake zimasiyanasiyana malinga ndi munthu, zomwe zimayambitsa matendawa ndi:

• Kusintha kwamitsempha, ndi kuthekera kwa kusowa kwamaganizidwe;
• Hepatosplenomegaly, yomwe ndi kukulitsa kwa chiwindi ndi ndulu, zomwe zimapangitsa kukulitsa pamimba;
• Kuuma olowa;
• Wakhama komanso wopanda nkhope, wokhala ndi mutu wawukulu, mphuno yayikulu ndi milomo yolimba mwachitsanzo;
• Kutaya kwakumva;
• Kusinthika kwa diso;
• Kuvuta kusuntha;
• Matenda opatsirana pafupipafupi;
• Kulankhula kovuta;
• Kuwonekera kwa zotupa pakhungu;
• Kukhalapo kwa hernias, makamaka umbilical ndi inguinal.

Pazovuta kwambiri pangakhalenso kusintha kwa mtima, kuchepa kwa mtima, komanso kupuma, zomwe zingayambitse kutsekeka kwa ndege ndikuwonjezera mwayi wamatenda opumira, omwe atha kukhala owopsa.

Chifukwa chakuti zizindikiritso zimawonekera ndikusintha mosiyanasiyana pakati pa odwala omwe ali ndi matendawa, chiyembekezo chokhala ndi moyo chimasinthanso, ndi mwayi wambiri wakufa pakati pa zaka khumi ndi ziwiri zoyambirira za moyo pomwe zizindikilozo zimakhala zowopsa.

Momwe matendawa amapangidwira

Kuzindikira kwa Hunter Syndrome kumapangidwa ndi wamajini kapena dokotala wamba malinga ndi zomwe munthuyo adapeza komanso zotsatira za mayeso ena ake. Ndikofunika kuti matendawa asapangidwe malinga ndi mawonetseredwe azachipatala, chifukwa mawonekedwe ake ndi ofanana kwambiri ndi a mucopolysaccharidoses ena, ndipo ndikofunikira kuti adotolo azilamula mayeso apadera. Dziwani zambiri za mucopolysaccharidosis ndi momwe mungazindikire.

Chifukwa chake, ndikofunikira kuyeza ma glycosaminoglycans mumkodzo ndipo, makamaka, kuti muwone kuchuluka kwa michere ya Iduronate-2-Sulfatase mu fibroblasts ndi plasma. Kuphatikiza apo, mayesero ena nthawi zambiri amalimbikitsidwa kuti aone kuopsa kwa zizindikilo, monga ultrasound, kuyesa kuyesa kupuma, audiometry, kuyesa kwamitsempha, kuyezetsa maso ndi kumveka kwa chigaza ndi msana, mwachitsanzo.

Chithandizo cha Hunter Syndrome

Chithandizo cha Hunter Syndrome chimasiyanasiyana kutengera mawonekedwe omwe anthu amapereka, komabe nthawi zambiri amalimbikitsidwa ndi adotolo kuti apange ma enzyme kuti ateteze kupitilira kwa matendawa ndikuwoneka kwamavuto.

Kuphatikiza apo, adotolo amalimbikitsa chithandizo chapadera cha zizindikilo zomwe amapatsidwa komanso chithandizo chantchito komanso kulimbitsa thupi kuti alimbikitse kuyankhula ndi kuyenda kwa odwala omwe ali ndi Syndrome kuti apewe zovuta zamagalimoto ndi zolankhula, mwachitsanzo.